Congenital complete atrioventricular conduction block (CCAVB) is a rare congenital arrhythmia with an incidence of approximately 1 in 22,000 [1] and a high early mortality rate, but if diagnosed early and actively intervened can significantly reduce the mortality rate. Since some children with CCAVB lack obvious clinical symptoms in early stages and are easily overlooked until preschool or school age, it is necessary to retrospectively study the clinical characteristics and treatment regression of children with CCAVB to provide a clinical basis for early diagnosis, treatment, and improvement of prognosis, and we summarize and analyze the data of children hospitalized with CCAVB diagnosed between August 2000 and October 2010 in a hospital 1. Subjects All children were examined by electrocardiogram, cardiac echocardiogram, chest X-ray, Holter, etc. Fasting glucose, electrolytes, electroencephalogram and cranial CT examination were routinely performed in children with As attack to exclude other disorders. If a child with CAVB has a clear history of rheumatic fever, viral myocarditis or a combination of complex precordial heart, it should be excluded. (1) Diagnosis: Diagnostic criteria for CCAVB: CAVB diagnosed in the fetal period, at birth and in the neonatal period, older children need to exclude diseases that can cause cardiac conduction dysfunction such as diphtheria, rheumatic fever, congenital syphilis, etc.; some simple precordial diseases such as atrial septal defect (ASD), patent ductus arteriosus (PDA), myocardial ventricular defect (VSD) can be combined, but the combination of Complex precordial diseases such as complete endocardial cushion defect (CAVC), corrected large vessel dislocation (L-TGA), single ventricle (SV) and atrial heterogeneity need to be excluded [2]. All cases in this group were diagnosed by clinical physical examination, relevant biochemical tests, EKG, Holter, chest radiograph and echocardiography to exclude complex precocious and acquired causes, and family history and maternal pregnancy were consulted for all confirmed children. (2) Clinical features: Children in all age groups were mainly diagnosed with bradycardia, asystole or heart murmur. Children with CCAVB were evaluated based on heart rate, EKG, Holter and echocardiography, and activity tolerance. (3) Treatment: Children diagnosed with CCAVB need to have ECG, echocardiogram and Holter examination to assess the severity of their disease. Since the clinical symptoms are mainly related to the fast or slow ventricular rate and the mortality rate is mainly related to the age at the time of diagnosis, some children with ventricular rate in the normal range of the age group, without obvious clinical symptoms and aged >4 years can be followed up on an outpatient basis and parents should be informed of some precautions A permanent pacemaker should be considered in children with the following conditions: enlarged heart with cardiac insufficiency; history of asystole; prolonged QTc with tip-twisting ventricular tachycardia confirmed by Holter; significantly reduced activity tolerance; RR >3 seconds maximum during wakefulness and >5 seconds during sleep; ventricular rate in the neonatal period.